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Biomedical subjects

H Frisch

Publications and source records attributed to H Frisch.

At least 109 records · Page 6Linked to original sources
PubMed1986

[Increased prolactin concentration and thyrotropic insufficiency in patients with growth hormone deficiency].

An increase in TSH secretion in patients with growth hormone deficiency (GHD) indicates hypothalamic thyrotropic dysfunction. A simultaneous increase in prolactin (PRL) response to TRH is often observed in these patients. In order to find out whether these patients need additional thyroxine treatment, we investigated thyrotropic function in 15 patients with GHD and increased PRL secretion. 3 groups of patients emerged: 1. Four patients were clinically hypothyroid (T42.9 +/- 1.3 micrograms/dl); 2. Four patients developed low T4 levels during growth hormone therapy (T43.5 +/- 0.1 micrograms/dl), although their T4 levels were normal before growth hormone treatment was initiated (T45.7 +/- 0.5 micrograms/dl). 3. Seven patients had T4 levels in the lower normal range (7.0 +/- 0.8 micrograms/dl). The growth velocity of these patients was monitored when thyroxine treatment was given in addition to hGH. Thyroxine administration resulted in suppression of TSH secretion in all patients while PRL concentrations remained elevated. Growth velocity was improved with additional T4 therapy in groups 2 and 3 without undue acceleration of bone age. All patients with GHD and elevated PRL levels should be carefully monitored for thyrotropic dysfunction.

Body Height↗
PubMed1986

Congenital hypothyroidism in a Turkish family: the role of immunoglobulins blocking the trophic effects of TSH and maternal-foetal relationship.

A Turkish family with frequent intermarriages is described, in which two siblings were born with persistent forms of congenital hypothyroidism, in the elder child concomitant with absent radioactive thyroid imaging. The mother was clinically euthyroid throughout the period of observation, but showed in addition to thyroid microsomal antibodies, high levels of immunoglobulins blocking the trophic action of TSH. These maternal growth blocking antibodies were transiently present in the youngest of the siblings (from birth to 2 months of age). She had a relatively mild form of congenital hypothyroidism (T3: 33 micrograms/100 ml; T4: 3.9 micrograms/100 ml). The older sibling, with proven non-functioning thyroid tissue (negative thyroidscan, T4: 0.4 microgram/100 ml) produced the growth-blocking immunoglobulins herself and may thus represent a juvenile form of thyroid autoimmunity with a very early onset. An aunt and uncle of the children, both hypothyroid since birth, were at the age of 19 and 18 years weakly positive for growth blocking immunoglobulins. This study indicates that familial forms of congenital hypothyroidism are probably complex and may be brought about by maternal to foetal passage of thyroid reactive autoantibodies, but also by the inheritance of a trait for thyroid autoimmunity. In some cases these two mechanisms might act in conjunction.

Adolescent↗
PubMed1986

Serum melatonin is not affected by glucocorticoid replacement in congenital adrenal hyperplasia.

Recently a hypothesis has been proposed suggesting a negative feedback in the regulation of cortisol (F) and melatonin (Mel). To study a possible influence of F on Mel regulation we examined 13 children with congenital adrenal hyperplasia (CAH) on two occasions: once 3 days after cessation of F substitution (group 1; n = 13) and once during treatment with dexamethasone (1 mg/m2/day) and fludrocortisone (0.1 mg/m2/day) (group 2; n = 11) 11 children matched by sex and age served as controls (group 3). While serum 17 OH-progesterone levels, an indicator for the activation of the hypothalamo-pituitary-adrenal axis in CAH, were significantly (P less than 0.001) elevated in untreated patients (group 1), serum Mel levels were not different among the 3 groups nor was the diurnal secretion pattern of Mel affected. Nocturnal serum Mel concentrations, however, correlated with the age of the subjects (r = 0.55, P less than 0.001 at 23.00 h), displaying high values in early childhood that declined with progressing age. The presented data do not support the view of a classical feedback mechanism in the regulation of Mel and F in humans. However, it confirms the description of a tremendous fall of nocturnal Mel concentrations during childhood.

17-alpha-Hydroxyprogesterone↗
PubMed1986

Growth hormone deficiency due to GH-N gene deletion in an Austrian family.

An 11 year old Austrian boy with isolated growth hormone deficiency type I A is described. On institution of GH therapy at the age of 2 2/12 years there was only a short growth response and anti-GH-antibodies with high binding capacity were detected, and growth was inhibited. Examination of the nuclear DNA by restriction endonuclease analysis demonstrated a defect of the GH-N gene in the patient. The results suggest the deletion in this Austrian family is different from that seen in other patients. The parents were heterozygous for the deletion and had a subnormal GH response to stimulation with arginine, but their somatomedin-C concentrations and their heights were normal. The patients' sister was of normal height, hormone analyses were normal, and the GH-N gene was not affected.

Antibodies↗
PubMed1986

[Spinal lipoma with a dural closure defect as a cause of neurogenic bladder and chronic renal failure].

It is reported on a 6-year-old boy, in whom 3 years after the appearance of a neurogenic disturbance of the urinary bladder a lipoma in the spinal canal of the inferior thoracic region was diagnosed myelographically. The operative removal of the growing and displacing fatty tissue which by a (congenital?) dural gap continued in epidural direction indeed resulted in a far-reaching regression of the paresis of the lower extremities, not, however, in an improvement of the urological picture of the disease. The renal insufficiency caused by the hydronephrosis was no more reversible, which emphasizes the importance of the early diagnosis of this relatively infrequent malformation.

Child↗
PubMed1986

[Pharmacokinetic-therapeutic studies of ceftriaxone in premature and mature newborn infants].

Ceftriaxone a third generation Cephalosporine exhibits a high degree of antimicrobial activity against the most common pathogens causing life threatening infections in premature and newborn infants. Ceftriaxone was used therapeutically in 16 premature and newborn infants with proven or suspected bacterial infections. Pharmakokinetic investigations were performed during this therapeutic trial. 0.1 ml of blood was taken at 2, 6 and 10 hours after intravenous administration of 50 mg/kg BW Ceftriaxone administered as a single daily bolus injection. Again on day two and four 2 hours after readministration of the same dose, serum concentrations were determined by a biologic test method. With these five samples only, we were able to calculate all clinically relevant pharmakokinetic parameters. There was a high degree of agreement between the experimentally determined and the calculated parameters. Premature infants showed a lower Cmax (115 micrograms/ml) which corresponded to the higher volume of distribution of 44% in this age group. Newborn infants in contrast showed a Cmax of 129 micrograms/ml corresponding to a volume of distribution of 39%. The halflife of elimination was 10.4 and 9.6 hours resp. for premature and mature newborn infants. Cumulation of the drug was seen during the first two days of treatment. A steady state however ensued on day three in both age groups after which no further increase in maximum serum concentrations was seen. Our data suggest, that 50 mg/kg BW once daily given intravenously by bolus-injection or short infusion over 30 minutes constitutes sufficient therapy for serious bacterial infections in premature and newborn infants with susceptible organisms.(ABSTRACT TRUNCATED AT 250 WORDS)

Bacterial Infections↗
PubMed1986

Cri du chat-syndrome in combination with partial trisomy 9 p.

A partial monosomy 5p leading to the Cri du chat-Syndrome combined with a partial trisomy 9p was observed in a mentally defective boy with typical clinical features for both syndromes. This chromosomal aberration is inherited from a t [5; 9] (p. 13.3; 13.1) translocation carrier father. Further family investigations showed many balanced translocation carriers through several generations.

Abnormalities, Multiple↗
PubMed1985

Capillary gas chromatography as a tool for characterization of urinary steroid excretion in patients with congenital adrenal hyperplasia.

Urinary steroid excretion was studied by capillary gas chromatography in 23 patients with congenital adrenal hyperplasia. In 5 patients the estimated excretion rates of pregnanetriol were in or below the normal range and 7 patients presented supranormal excretion rates of tetrahydro-cortisone and/or other glucocorticoid metabolites. Deficiency of 21-hydroxylase was nevertheless demonstrated in each patient by an increased ratio of excreted precursors vs products of 21-hydroxylase, e.g. of pregnanetriol/tetrahydro-cortisone. Due to this relative deficiency of glucocorticoids the patients' steroid excretion was further characterized by a predominance of 5 alpha-hydrogenated C19O3 metabolites (11-keto-androsterone, 11-hydroxy-androsterone) over their 5 beta-hydrogenated homologues (11-keto-etiocholanolone, 11-hydroxy-etiocholanolone). An apparent preponderance in the excretion of pregnenetriol over that of pregnanetriol was found in 4 patients, but the presence of pregnenetriol was not confirmed by mass spectrometry following prepurification of the urine samples by thin-layer chromatography indicating interference of an unidentified steroid metabolite with the initial gas chromatographic analysis. The simultaneous determination of steroids serving as precursors or products of 21-hydroxylase by capillary gas chromatography helps to establish the diagnosis of 21-hydroxylase deficiency and to characterize the pattern of steroid excretion in this syndrome even in patients where the estimation of single urinary steroids may lead to erroneous conclusions.

Adolescent↗
PubMed1985

Apolipoproteins and lipoproteins in children with type I diabetes: relation to glycosylated serum protein and HbA1.

Serum levels of cholesterol (C), triglycerides (TG), lipoprotein-C and apolipoproteins (apo) A-I, A-II and B were measured in 30 children with type I diabetes mellitus (16 boys, 14 girls, aged 11-14 years) and in 26 healthy controls (15 boys, 11 girls, aged 10-13 years). For 19 diabetics controls matched for age, sex and relative body weight were selected. The diabetic patients were considered to be in fair metabolic control according to HbA1 levels and glycosylated serum protein concentrations. Mean serum apo A-I, A-II and B, C, TG, low density lipoprotein cholesterol (LDL-C) and high density lipoprotein cholesterol (HDL-C) did not differ significantly between diabetic nondiabetic children. Very low density lipoprotein cholesterol (VLDL-C) was significantly higher in diabetic children than in controls. Serum C and LDL-C levels showed close univariate linear correlations with glycosylated serum protein (LDL-C: r = 0.53, p less than 0.01, C: r = 0.58, p less than 0.01) in diabetics. The ratio LDL/HDL-C was significantly correlated to HbA1 levels (r = 0.47, p less than 0.01). By canonical and multiple linear correlation analysis significant relations of a selected set of variables concerning the control and therapy of diabetes (serum glucose, HbA1, glycosylated serum protein, insulin dose) with a set of lipoprotein variables (C, TG, VLDL-C, HDL-C, LDL-C, apo A-I, A-II, B) could be demonstrated. From these data we conclude that significant relations between atherogenic serum lipids and lipoproteins (C, LDL-C) and the degree of metabolic control exist in diabetic children, even in the absence of marked dyslipoproteinemia.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1985

[Percutaneously implanted silastic catheters in the neonate].

In 21 infants requiring total parenteral nutrition 23 Silastic central venous catheters were placed by percutaneous insertion into the basilic vein. Weight of the infants was 1770 g (980-3540 g), age at time of insertion 1,6 days (1-16 d), gestational age 32 weeks (29-39) weeks. Catheters remained in place between 3 and 37 days, total 451 patient days. No problems as infection or thrombophlebitis or caval obstruction occurred.

Catheters, Indwelling↗
PubMed1985

[Development of premature children below a birth weight of 1,500 g].

5,928 newborn infants were admitted between the years 1974 and 81 to the University of Innsbruck, Department of Pediatrics. 418 (7%) of them were premature infants with a birthweight of less than 1,500 g. 21 (18%) of the premature infants with a birthweight below 1,000 g survived (21 of 117). Among those 21 surviving infants only 7 (33%) had an appropriate birthweight, 14 (67%) were too small for their gestational age. Survival in the weight range between 1,000-1,500 g was 71% (213 of 301). The percentage of dystrophic infants was 23% (49 of 213). The place of birth has a significant influence on the survival of these very small immature infants. 24% (n = 84) of patients delivered in the nearby Clinic of Obstetrics survived in contrast to only 3% (n = 33) of those born in peripheral hospitals. This is reemphasized by the evaluation of psychomotor development of surviving infants; of 14 patients with normal psychomotor milestones 13 were delivered at our obstetric department. 157 patients were regularly followed up during their first year of life. 43% can be considered normal by all parameters. 57% show some abnormalities in their development during the first years of life. During their second year of life only 13% showed psychomotor problems and this number decreased to 5% in the third year. All these children show varying degrees of hemi- or diplegia and/or seizure activities. A routine check up at 12 month of life seems important as all patients with permanent retardation can be correctly diagnosed at this age.(ABSTRACT TRUNCATED AT 250 WORDS)

Body Height↗
PubMed1985

[Unusual cutaneous emphysema in a newborn infant following bilateral pneumothorax].

We report a patient with subcutaneous emphysema after a bilateral pneumothorax of gigantic extent: Subcutaneous emphysema extended over the chest, neck, axilla and both arms. The scalp was severed from the galea by an enormous air-cushion. Theories about the pathogenetic mechanisms are discussed. Therapy consisted of treatment of the underlying condition e. g. thoracocentesis with pleural drainage and artificial ventilation. The subcutaneous emphysema resolved without further therapeutic measures; only the subgaleal air was removed by aspiration.

Emphysema↗
PubMed1985

[Mechanism of a genetically conditioned failure to thrive shown in a patient with ring chromosome 18].

The influence of genetic factors on growth is only partially understood. The assumed regulatory mechanism is an interplay of multiple genes, which are localized on various chromosomes. Numerical and unbalanced structural chromosome anomalies cause abundance or lack of genes and gene products. As a consequence the subtle, in their complexity hardly fully conceivable regulatory mechanisms for metabolism and growth of the single cells appear to be disturbed. This kind of model is supported by the occurrence of growth failure in most numerical and structural chromosome anomalies. Further evidence is the variability of the phenotype in cases of ringchromosome 18, which depends on the localization and degree of loss of chromosome material preceding ring formation: depending on the participation of one or several growth-regulating genes normal or impaired growth follows. Consequently we find some normally thrived proponents within the group of predominantly mal grown people with ringchromosome 18. Besides growth the phenotypical variability is concerned with a whole lot of body-functions and systems and virtually every case reported in the literature shows individual signs. This is also true for the patient reported in this paper, in whom we additionally describe the following hitherto not mentioned signs: rudimentary pair of first ribs, apical pulmonary hernias, submammilary dermal groove, hemangioma, meatal stenosis of the urethra, unilateral kidney aplasia, 6 lumber vertebral bodies, umbilical-, abdominal- and inguinal hernias.

Abnormalities, Multiple↗
PubMed1985

[Effect of diabetes mellitus on the development of personality and behavior of children and adolescents].

A questionnaire was developed to determine the effects of being afflicted with diabetes mellitus upon the conduct and personality of juvenile type I diabetics. The following subjects were dealt with: desire for independence, feeling of being (over) protected (dependence) by the parents or their substitutes, social contact to children of the same age, social contact to adults, general mood. This questionnaire was filled out by 87 children (35 diabetics and a control group of 52 healthy children) aging 9-15 years. We did not observe any signs of a different development among diabetics, apart from their social contact to adults.

Adaptation, Psychological↗
PubMed1984

[Psychosocial dwarfism--a rare form of growth disorder].

Psychosocial dwarfism is a syndrome caused by emotional deprivation (maternal deprivation), characterized by symptoms of delayed motor and intellectual development, abnormal eating and drinking habits, enuresis and encopresis, aggressiveness and a pathological family structure. Diagnosis of psychosocial dwarfism is easy if the case history is carefully elicited and the growth hormone level is determined within the first few days following change in environment (e.g. hospitalization). Difficulty in reaching the correct diagnosis or misdiagnosis can occur if the symptomatology is not studied in its entirety. Diagnosis at the earliest possible stage is very important for the further development of the child, since behavioural disturbances and growth retardation are reversible with environmental change. This is demonstrated by the presentation of the case history of a 6 year-old boy--the third case reported in the German literature.

Child↗
PubMed1984

Fall in nocturnal serum melatonin during prepuberty and pubescence.

Morning (7:30 AM to 10:00 AM) and nighttime (11:00 PM to 1:00 AM) serum melatonin concentrations were measured in 89 children, adolescents, and young adults. Morning levels (generally 0-20 pg/ml) did not change with sexual maturation or with age. Nighttime levels decreased significantly both with sexual maturation and with age. Nighttime serum melatonin fell from 195 +/- 24 pg/ml (mean +/- SEM) in prepubertal children younger than 7 years of age, to 119 +/- 23 pg/ml in prepubertal children aged 7 years or older, to 49 +/- 4 pg/ml in young adults (puberty stage V). Similarly, nocturnal serum melatonin levels fell from 210 +/- 35 pg/ml in the youngest age group (ages 1-5) to 133 +/- 17 in children aged 5-11 years and to 46 +/- 4 in young adults. Nocturnal plasma concentrations of luteinising hormone measured at various stages of puberty tended to vary inversely with those of melatonin (r = -0.35). Past difficulties in demonstrating a relation between gonadal maturation and human pineal function may have reflected the use of insufficiently sensitive or specific melatonin assays, or serum sampling only during daytime, or the initiation of sample collection when subjects were already too old.

Adolescent↗